The Role of the Bicuspid Aortic Valve in Sudden Cardiac Death – Findings at Cardiac Autopsy

Nikhil Chatrath, Joseph Westaby, Gherardo Finocchiaro, Sanjay Sharma, Maite Tome Esteban, Michael Papadakis, Mary N Sheppard. Cardiovascular pathology: the official journal of the Society for Cardiovascular Pathology. 2023 Feb 22. Read the paper here


Background: Bicuspid aortic valve (BAV) is the most common congenital cardiac defect in the adult population, with a prevalence of 0.5-2%. It is well recognised that aortic stenosis (AS), aortic regurgitation (AR) and aortopathy may develop by the fifth or sixth decade of life. There is a paucity of autopsy studies evaluating the hearts of subjects with BAV. The aim of this study is to help to ascertain the role of the bicuspid valve in the cause of sudden cardiac deaths.

Methods: A database of 6325 whole hearts referred to a specialist cardiac pathology centre between 2004 and 2021 was reviewed to identify a subgroup of 91 subjects with a BAV reported. All cases had a negative full body autopsy and toxicology before being referred and subsequently underwent detailed cardiac evaluation including histological analysis by expert cardiac pathologists.

Results: The mean age of death was 37±16 years (84% male). Death was attributed to aortic valve or aortic disease in 57% (n=52) of cases; AS 30% (n= 27), endocarditis 11% (n=10), aortic dissection (AD) 9% (n=8) and AR 8% (n=7). In the remaining 43% of cases, BAV was an incidental finding.

Conclusion: The majority of deaths in young individuals with BAV were attributed to complications related to the aortic valve or aorta indicating that BAV is not a benign condition. When a BAV is identified, individuals should be appropriately follow-up with imaging to inform the optimal timing of intervention before a complication develops that may predispose the individual to a premature death.